UNLABELLED: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), also termed Hashimoto's encephalopathy (HE), is a rare immune-mediated disorder and is also affecting children and adolescents. It is characterized by altered mental status, seizures, and cognitive dysfunction. Therapeutic options include steroid treatment and prognosis range from complete recovery, a relapsing course to long-term cognitive sequelae. We describe a previously healthy 13-year-old girl presenting to the emergency room with coma and refractory status epilepticus. Generalized tonic-clonic seizures persisted after pre-hospital infusion of antiepileptic medication. She was found to have highly elevated levels of thyroid-stimulating hormone and anti-thyroid peroxidase antibodies not only in blood but also in cerebrospinal fluid while showing negative results for traumatic, infectious, metabolic, toxic, neoplastic, or other known specific autoimmune diseases. Cranial neuroimaging revealed no abnormality. A diagnosis of SREAT was established, and the patient improved rapidly on corticosteroids and levothyroxine therapy. However, 3 months after the discontinuation of steroid treatment, the girl relapsed. The current literature regarding SREAT is reviewed and summarized. CONCLUSION: In children with SREAT, early diagnosis and treatment with corticosteroids is crucial and can lead to rapid clinical improvement. Clinicians should be aware of this uncommon but treatable condition, especially in female adolescents with unexplained seizures or an encephalopathic state.
UNLABELLED: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), also termed Hashimoto's encephalopathy (HE), is a rare immune-mediated disorder and is also affecting children and adolescents. It is characterized by altered mental status, seizures, and cognitive dysfunction. Therapeutic options include steroid treatment and prognosis range from complete recovery, a relapsing course to long-term cognitive sequelae. We describe a previously healthy 13-year-old girl presenting to the emergency room with coma and refractory status epilepticus. Generalized tonic-clonic seizures persisted after pre-hospital infusion of antiepileptic medication. She was found to have highly elevated levels of thyroid-stimulating hormone and anti-thyroid peroxidase antibodies not only in blood but also in cerebrospinal fluid while showing negative results for traumatic, infectious, metabolic, toxic, neoplastic, or other known specific autoimmune diseases. Cranial neuroimaging revealed no abnormality. A diagnosis of SREAT was established, and the patient improved rapidly on corticosteroids and levothyroxine therapy. However, 3 months after the discontinuation of steroid treatment, the girl relapsed. The current literature regarding SREAT is reviewed and summarized. CONCLUSION: In children with SREAT, early diagnosis and treatment with corticosteroids is crucial and can lead to rapid clinical improvement. Clinicians should be aware of this uncommon but treatable condition, especially in female adolescents with unexplained seizures or an encephalopathic state.